Ebstein's anomaly is an uncommon congenital heart defect characterized by an abnormal tricuspid valve. Pregnancy can be well tolerated, although cyanosis, arrhythmias, and cardiomegaly are associated with poor prognosis. An 18-year-old woman with a history of congenital cardiomyopathy presented at 37 weeks of gestation with hypoxia, dyspnea, central cyanosis, and uterine contractions. The patient underwent emergent cesarean delivery. Echocardiography revealed Ebstein's anomaly with patent foramen ovale and right-to-left shunting. Tricuspid valvuloplasty with primary closure of patent foramen ovale was performed. This case is presented, accompanied by a review of the literature.